We report on a patient with persistent mullerian duct syndrome and normal external genitalia who had embryonal cancer of the testis 16 years after neonatal bilateral orchiopexy. In previous cases the testes of these patients have not been considered predisposed to form tumors. However, the occurrence of a testis tumor has been reported in 8 patients with this syndrome. The specific factors resulting in tumor formation in such patients are uncertain. Until they are clarified we suggest that these patients should be observed carefully for the possible development of testis tumor.
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